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Biomedical subjects

G M Palmieri

Publications and source records attributed to G M Palmieri.

At least 19 recordsLinked to original sources
PubMed1991

Effect of dantrolene in Duchenne muscular dystrophy.

We have demonstrated that maneuvers capable of reducing Ca influx into cells have beneficial effects in dystrophic hamsters and Duchenne muscular dystrophy. Since dantrolene inhibits Ca release from the sarcoplasmic reticulum, its effects on DMD was studied in 7 patients of 6 to 13 years of age (mean 10.8 years). Patients were studied for 4 years with tri-monthly evaluations of manual muscle testing (MMT), functional activity, and serum CK and aldolase. During the first 2-year period, no medicines were given and served as control. In the second 2-year period, dantrolene 8 mg/kg/d was administered. No side effects were observed. In 1 patient, mild weakness occurred that disappeared when the dose was reduced to 6 mg/kg/d. The 95% confidence limit for the difference in slopes of regression lines from tri-monthly MMT was asymmetric in favor of dantrolene in 5 of 7 patients. Serum CK did not differ between the first and second year of the control and treatment periods, respectively. However, it fell significantly from the second year of control to the first year of treatment (P = 0.003). The fall during the first year of treatment was significantly greater (P less than 0.01) than in age-matched natural history controls during the same length of observation. There was a 3-fold reduction in CK when the pooled values of the first and second year control vs. treatment periods were analyzed. No changes were observed in functional activity and serum aldolase. The data suggest that dantrolene reduces serum CK in DMD associated with a lessening trend in MMT deterioration.

Adolescent↗
PubMed1991

Black bones following long-term minocycline treatment.

During a surgical procedure, black vertebrae were observed in a 42-year-old white woman. An undecalcified iliac crest bone biopsy specimen revealed intense fluorescence compatible with tetracycline labeling and osteoporosis. A urinary screening test was negative for amino acids. The patient had been treated with minocycline hydrochloride (100 to 300 mg/d) for at least 6 years. Since minocycline is known to discolor many body tissues, it is likely that the black discoloration of bone in our patient was caused by the long-term intake of the antibiotic.

Acne Vulgaris↗
PubMed1990

The effect of diltiazem on calcinosis in a patient with the CREST syndrome.

We describe a patient with a 23-year history of progressive calcinosis and features of the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) who was treated with diltiazem, 240 mg/day, for 5 years. No clinical exacerbation of calcinosis occurred during treatment. Radiographs showed no new lesions, and there was reduction in the size of the existing lesions. Bone scans revealed a progressive decrease in the uptake of the radionuclide by soft tissue foci. We propose that diltiazem may stop the progression of calcinosis by reducing the cellular calcium influx in affected tissues.

Administration, Oral↗
PubMed1989

Effect of calcitonin and vitamin D in osteoporosis.

Vitamin D has complex effects in bone: it stimulates matrix formation and bone maturation but also enhances osteoclastic activity and may influence differentiation of bone cell precursors. Calcitonin inhibits the function of osteoclasts, reducing bone resorption, thus, the combination of vitamin D and calcitonin could result in a positive bone balance. We tested the hypothesis that chronic treatment with high doses of vitamin D (150,000 U/week), moderate doses of salmon calcitonin (120 MRC U/week), and adequate Ca supplementation (1 g/day) could be beneficial in osteoporosis. Thirteen women with postmenopausal osteoporosis received this treatment for 2-6 years (mean 3.5 years). No side effects, hypercalcemia, or hypercalciuria occurred. There was marked reduction in bone pain. The fracture rate in 11 patients with vertebral compression fracture was 240/1,000 patient years, threefold lower than the reported 834 fractures for untreated patients of similar age. Single photon bone densitometry of the radius did not change. Iliac crest bone biopsies obtained at the initiation and conclusion of the study showed a 43% increment in trabecular bone volume (P = 0.0003), without changes of the normal osteoid thickness, surface, and volume. Because single photon densitometry reflects mostly cortical bone, the data suggest that the combination of vitamin D and calcitonin increases trabecular bone mass and prevents the fall of cortical bone mass in osteoporosis. Previous reports suggest that calcitonin alone or with small doses of vitamin D increased bone mass for about 2 years. The present study suggests a prolonged beneficial effect of the combination of high doses of vitamin D with rather moderate (less than 150 MRC U/week) doses of calcitonin in postmenopausal osteoporosis.

Aged↗
PubMed1989

Oral calcium chloride in hypoparathyroidism refractory to massive doses of calcium carbonate and vitamin D.

Surgically induced hypoparathyroidism often responds satisfactorily to intravenous Ca administration, oral CaCO3 and vitamin D2. A 17-year-old girl developed hypoparathyroidism following partial thyroidectomy for thyrotoxicosis. Hypocalcemia was refractory to treatment with massive doses of vitamin D2, up to 150,000 U, 3-6 gm of oral Ca as CaCO3 and 2 micrograms of 1,25-dihydroxycholecalciferol per day. Intravenous Ca gluconate (360 mg of elemental Ca/d, in divided doses) was needed to correct tetany. After 25 days of unsuccessful therapy, oral administration of 30 ml of a 10% solution of CaCl2 (1.09 gm of elemental Ca) was followed by normalization of serum Ca (8.9 mg/dl) within 7 hours. This dose was repeated every 8 hours for 6 days and oral CaCO3 and IV Ca gluconate were discontinued. Serum Ca remained within normal range but hyperchloremic acidosis developed. This was corrected by providing, in addition to vitamin D, 2 g/d of Ca supplementation, 1 gm in the form of 10% CaCl2 solution and 1 gm as CaCO3 in two doses given simultaneously. During 12 months of observation, serum Ca, P and Cl have been consistently within normal limits. This patient was found to have achlorhydria, unresponsive to normalization of thyroid function and serum Ca. These findings indicate that refractoriness to oral CaCO3 and vitamin D may be caused by achlorhydria. Oral administration of CaCl2 solution can promptly correct this defect. Monitoring of serum Cl and CO2 is needed to avoid hyperchloremic acidosis.

Administration, Oral↗
PubMed1989

Pamidronate sodium and calcitonin-resistant Paget's disease. Immediate response in a patient.

One three-day course of intravenous pamidronate sodium (3-amino-1-hydroxypropylidene-1,1-bisphosphonate), 30 mg/d, in a patient with calcitonin-resistant Paget's disease resulted in the following: marked clinical improvement within two weeks; normalization of urinary hydroxyproline value; fall of serum alkaline phosphatase value (900 to 250 U/L); a rise in serum osteocalcin value by the tenth week that returned to pretreatment levels in the 16th week; transient hypocalcemia with elevation of parathyroid hormone value; reduction in urinary calcium excretion; and improvement in bone scans. No adverse reactions occurred, with the exception of mild and transient hyperpyrexia for 48 hours during pamidronate administration. White blood cell counts did not change and serum interleukin 1 was undetectable before and after treatment with pamidronate. Pamidronate seems to be highly effective in the treatment of Paget's disease of the bone, but its profound effects on mineral and bone metabolism require close monitoring during the short-term period of intravenous treatment.

Alkaline Phosphatase↗
PubMed1988

Effect of chronic treatment with the calcium antagonist diltiazem in Duchenne muscular dystrophy.

We conducted a double-blind trial with the calcium antagonist, diltiazem (8 mg/kg/d), for 24 to 32 months in 22 boys with Duchenne muscular dystrophy, who were paired by functional activity and age. No adverse clinical or ECG effects of diltiazem were detected. In eight matched pairs, completing 28 months, manual muscle testing scores fell somewhat less in the diltiazem group (from 5.5 to 4.6) than in the placebo group (from 5.3 to 4.2), although the difference between groups was not significant (p = 0.06). The 95% confidence interval for the difference in slopes of regression lines obtained from trimonthly manual muscle tests on all subjects was markedly asymmetric in favor of the diltiazem group, but this difference was also not significant. There was less deterioration of functional activity of lower extremities in the diltiazem-treated group, when beginning and end values were analyzed (p = 0.03). However, the difference in slopes of regression lines obtained from trimonthly determinations was nonsignificant. Similarly, the beginning versus end comparisons of systolic and diastolic blood pressure showed a significantly (p less than 0.05) smaller elevation of blood pressure in the diltiazem-treated group, but no difference was observed when the slopes of all values were analyzed. All other clinical and laboratory variables were unaffected by diltiazem treatment. The findings in manual muscle tests and functional activity suggest a beneficial trend with chronic diltiazem treatment in DMD.

Adolescent↗
PubMed1988

Hypercalcemia.

Explore the source record for details and available documents.

Aluminum↗
PubMed1986

Duchenne muscular dystrophy: normal ATP turnover in cultured cells.

We examined ATP metabolism in cultured muscle cells and fibroblasts from patients with Duchenne dystrophy. ATP and ADP levels were the same in cultured cells from normal subjects and patients and there was no difference in ATP synthesis or degradation. Although there was a significant decrease in radioactively labelled ATP after incubation with deoxyglucose in Duchenne muscle cells, there was no difference in ATP concentration or ADP metabolism.

Adenine Nucleotides↗
PubMed1986

Skeletal surveys in multiple myeloma. Radiologic-clinical correlation.

Thirty-three patients with multiple myeloma were studied with serial skeletal surveys, serum immunoglobulin levels, and postabsorptive urinary hydroxyproline (Spot-HYPRO) determinations. Twenty receiving chemotherapy were also followed with skeletal surveys in order to evaluate bone response to treatment. A close association was found between skeletal findings and changes in immunoglobulin levels with positive correlation in 71% of the patients. A similar association was found between skeletal disease and Spot-HYPRO level changes in 65%. Five of 12 patients (42%) with partial or complete clinical response to chemotherapy, demonstrated improvement in the appearance of skeletal lesions. Positive correlation between the roentgenographic changes and clinical markers of myeloma as well as therapeutic response, indicates that skeletal surveys are useful and effective in monitoring patients with multiple myeloma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Biliary excretion of aluminum in aluminum osteodystrophy with liver disease.

Two patients with chronic liver disease developed elevated serum aluminum concentrations and biopsy-proven osteodystrophy. Neither patient had chronic renal failure but both had received aluminum-containing antacids for long periods. We measured biliary and urinary aluminum excretion during antacid loading in patients with normal liver function. Our studies show that biliary excretion is an important route of elimination of orally absorbed aluminum, and we suggest that long-term antacid therapy in patients with severe liver disease be monitored with periodic serum and urinary aluminum determinations to avoid aluminum osteodystrophy.

Adult↗
PubMed1985

Chronic allopurinol and adenine therapy in Duchenne muscular dystrophy: effects on muscle function, nucleotide degradation, and muscle ATP and ADP content.

Prompted by the controversy on the efficacy of allopurinol in Duchenne muscular dystrophy and by our observations of an abnormal adenine nucleotide turnover in this disease, we conducted an 18 month, double-blind clinical trial with allopurinol and adenine in 14 Duchenne boys paired according to age and functional activity. Detailed clinical evaluation was performed trimonthly. Muscle ATP and ADP content was measured before and after 1 year of treatment. The effect of therapy on adenine nucleotide turnover was determined. No significant difference was observed between the treated and placebo groups, but both showed a significant deterioration (p less than 0.05) in most clinical parameters. Muscle ATP was reduced in Duchenne dystrophy (p less than 0.02) but did not change with therapy, and no correction of the abnormal adenine nucleotide degradation was observed.

Adenine Nucleotides↗
PubMed1985

Duchenne muscular dystrophy: normal ATP turnover in cultured cells.

We examined ATP metabolism in cultured muscle cells and fibroblasts from patients with Duchenne dystrophy. ATP and ADP levels were the same in cultured cells from normal subjects and patients, and there was no difference in ATP synthesis or degradation. Although there was a significant decrease in radioactively labeled ATP after incubation with deoxyglucose in Duchenne muscle cells, there was no difference in ATP concentration or ADP metabolism.

Adenosine Triphosphate↗
PubMed1985

ATP degradation products after ischemic exercise: hereditary lack of phosphorylase or carnitine palmityltransferase.

We measured purine degradation products of ATP in plasma after ischemic exercise in eight normal subjects, one patient with myophosphorylase deficiency (McArdle's disease), and one with carnitine palmityltransferase deficiency. Normal subjects increase hypoxanthine and inosine, but not xanthine. Plasma purine levels were elevated above the normal range after ischemic exercise in McArdle's disease and in carnitine palmityltransferase deficiency after fasting. Those changes implied abnormally accelerated ATP degradation in these two myopathies.

Acyltransferases↗
PubMed1985

Soft tissue calcium and magnesium content in acute pancreatitis in the dog: calcium accumulation, a mechanism for hypocalcemia in acute pancreatitis.

The mechanism of hypocalcemia in acute pancreatitis remains unknown despite continued investigative work over the past several decades. Because acute pancreatitis is accompanied by multiple systemic manifestations and alterations of plasma membranes, the possibility that an abnormal translocation of calcium from extracellular to intracellular compartments could play a role in hypocalcemia of acute pancreatitis was investigated in dogs. Acute pancreatitis was induced by injecting bile into the pancreatic duct. Plasma calcium, magnesium, and amylase concentrations were determined. Calcium and magnesium contents were also measured in biopsy specimens of pancreas, liver, skeletal muscle, and kidney before and after induction of acute pancreatitis. As expected, hypocalcemia and hyperamylasemia occurred 6 hours after induction of pancreatitis, and persisted throughout the experiment, 13 to 25 hours. Plasma magnesium concentration fell at 6 and 18 hours, and returned to an almost normal level by the end of the study. A significant elevation in calcium content of pancreas (71%), liver (24%), and muscle (112%), and 25% reduction of calcium in kidney were observed in dogs with histologic signs of pancreatitis. However, tissue magnesium concentration fell in pancreas (18%) but remained unchanged in the other tissues investigated. No significant changes in any variables were detected in sham-operated animals. In another group of dogs in which the accessory pancreatic duct was not occluded when bile was injected, the histologic lesions were extremely mild, although the plasma calcium concentration and the pancreatic calcium and magnesium contents were altered just as much as in the severely affected dogs. Data suggest that the hypocalcemia of acute pancreatitis may be the result, at least in part, of accumulation of calcium in soft tissues. The decreased calcium content in kidney could be related to hypocalcemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Amylases↗